Bleeding Jejunal Varices and Portal Thrombosis in a Splenectomized Patient with Hereditary Spherocytosis

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Digestive Diseases and Sciences, Vol. 45, No. 2 (February 2000), pp. 373–377 CASE REPORT

MARCO BERTOLOTTI, MD, PAOLA LORIA, MD, PIETRO MARTELLA, MD, LUCIA CARULLI, MD, MARIO DE SANTIS, MD, and NICOLA CARULLI, MD KEY WORDS: hereditary spherocytosis; jejunal varices; portal thrombosis; splenectomy.

Bleeding from varices located in the small bowel is a loss and anemia; investigation of the gastrointestinal tract very uncommon finding; nonetheless, such events ac- by standard upper and lower endoscopy, performed at a company with a high mortality rate (1–4). Moreover, local hospital, had always been negative and so had the other routine imaging exams. Therefore, no definite diag- early diagnosis of jejunal or ileal varices cannot usu- nosis had been made, and the patient, with a suspected ally be accomplished with standard diagnostic tools diagnosis of erosive hemorrhagic gastritis, was put under (ie, esophagogastroduodenoscopy, colonoscopy). treatment with antisecretive drugs (ranitidine or omepra- Most reports in the literature relate to subjects with zole cyclically). The clinical history of the patient was oth- liver cirrhosis, often with hepatocarcinoma; in un- erwise unremarkable with the exception of a diagnosis of usual anatomical situations, varices may develop be- hereditary spherocytosis in childhood, leading to splenectomy at the age of 13. At the present time he was not taking yond the ligament of Treitz in adjunct to the far more any drugs. common location in the esophageal and gastric wall. On physical examination the patient was slightly over- Thrombosis of the portal vein is a common feature in weight, with no abdominal findings of clinical interest. The such conditions. Portal thrombosis has also been de- right leg had been moderately swollen, warm, and tender scribed in association with overt or latent myelopro- for the past five to six days; Doppler ultrasonography, liferative diseases (5); its occurrence in nonneoplastic performed upon admission, excluded the presence of deep venous thrombosis. Laboratory tests revealed mild hypo- hematological conditions in subjects with normal liver chromic microcytic anemia, with blood cell morphology function is quite uncommon. compatible with the diagnosis of hereditary spherocytosis, This report describes the observation of jejunal and marked thrombocytosis (800,000–900,000/␮l). Anti- varices, with repeated episodes of “melena of un- thrombin III was slightly lower than normal. Other values known origin,” some of which quite severe, as their were within the normal range. A bone marrow biopsy was clinical presentation in a patient with portal throm- performed and allowed us to rule out a primary hematological disorder other than spherocytosis. bosis and with otherwise absolutely normal liver func- Melena had resolved spontaneously two to three days tion, who had undergone splenectomy for hereditary after onset and even occult blood was undetectable in the spherocytosis in early adolescence. stool at the time of our observation. The patient felt sub- jectively well and the right leg pain and edema also subsided CASE REPORT spontaneously in a few days. Esophagogastroduodenoscopy had been performed the G.S., a Caucasian male 33 years of age, was admitted to week before admission, after the clinical appearance of our division nearly two weeks after an episode of melena. In melena and was negative, as were the previous ones. To rule the previous two and a half years he had presented two out a vascular anomaly of the small bowel, we performed a similar episodes, one of them leading to important blood jejunoenteroscopy, which showed the presence of submucosal venous varices beyond the ligament of Treitz (Figure 1); apart from their more distal location, they had the same Manuscript received February 26, 1999; accepted July 14, 1999. morphological appearance as esophageal varices, as the From the Dipartimento di Medicina Interna and Dipartimento endoscopist pointed out. A selective angiography of the di Scienze Mediche, Oncologiche e Radiologiche, Universita` degli Studi di Modena e Reggio Emilia, Italy. celiac and mesenteric arteries was subsequently performed; Address for reprint requests: Dr. Marco Bertolotti, Divisione di angiography of the superior mesenteric artery showed a Medicina III, Policlinico, Via del Pozzo 71, 41100 Modena, Italy. normal arterial phase, whereas the venous phase (Figure 2)

Fig 1. Enteroscopic imaging of submucosal varices in the proximal jejunum. revealed several venous dilatations in the territory of the sion and bleeding jejunal varices in subjects with superior mesenteric vein. The vein itself was not opacified, normal liver function, is extremely rare; a few reports whereas the portal vein was entirely thrombosed. The pic- have described small bowel varices after abdominal ture was confirmed at CT angiography (Figure 3). A diagnosis of portal hypertension with jejunal varices, surgery, usually at the site where an anastomosis was related to portal thrombosis, was made. Surgical devascu- performed (7). Interestingly, portal thrombosis was larization was proposed to the patient, who refused. also described to occur, independently of chronic liver disease, in subjects with hematological diseases, DISCUSSION in particular with myeloproliferative conditions (5, 8, Venous varices have been reported to occur at any 9). site of the gastrointestinal tract, even if location be- Hereditary spherocytosis is seldom associated with yond the duodenum is quite uncommon (1); their thrombotic risk (10). As far as we know, the only clinical presentation, though, may be quite serious report in the literature describing the association of and episodes of important intestinal hemorrhage hereditary spherocytosis and portal thrombosis is a have been described (2, 3). In the reports and reviews letter by McGrew and Avant (11); the patient de- of the literature (1, 3, 4), the finding is mostly asso- scribed developed portal thrombosis eight years after ciated with chronic progressive liver disease, often splenectomy, which became clinically apparent after complicated with hepatocarcinoma. In these clinical an episode of bleeding from esophagogastric varices. situations portal thrombosis is also relatively common We believe that in such patients more than one and formation of submucosal varices in extraesopha- factor may contribute to the development of portal geal sites may follow, or be associated with, the ap- thrombosis. One such factor is thrombocytosis sec- pearance of esophageal and/or gastric varices. In ondary to splenectomy; even if this condition is not some instances extraesophageal varices were detected usually associated with a hypercoagulable state, this after shunt operations to relieve portal hypertension might be favored by the association of hemolysis and (6). spherocytosis per se. Secondly, removal of the spleen Occurrence of portal thrombosis, portal hyperten- and of the splenic vein is likely to slow down portal

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Fig 2. Selective angiography of the superior mesenteric artery: venous phase showing gross jejunal varices (arrows); the superior mesenteric and portal veins are not opaciﬁed.

flow and to facilitate the onset of a thrombotic pro- tation at the right leg, which might have represented cess. Important hemodynamic changes take place af- a minor episode of venous thrombosis, in spontane- ter splenectomy or splenorenal shunt (12, 13). Portal ous resolution when Doppler sonography was per- thrombosis was indeed described after surgical sple- formed. Indeed venous thrombosis may be the cause nectomy in subjects with idiopathic or cirrhotic portal of leg ulcers, a quite common finding in patients with hypertension (14) and shortly after laparoscopic sple- spherocytosis. nectomy (15). The appearance of varices in the mesenteric small In our patient, bone marrow examination excluded bowel probably reflects an uncommon anatomical a primary hematological disorder other than sphero- situation where venous blood flow at relatively high cytosis. Nonetheless, even in the absence of overt pressure cannot drain through the coronary gastric myeloproliferative disease, portal thrombosis has and azygous veins due to the vascular deconnections been described in association with the tendency to of splenectomy or to extensive vascular involvement form “spontaneous” erythroid colonies in cultures of of the thrombotic process. A more limited extension bone marrow taken from the subjects (5). of thrombosis was probably present in the only other A prothrombotic state might be present in patients case reported in the literature (11), with a different with hemolytic syndromes, or in subjects with “occult” hemodynamic condition leading to the more usual myeloproliferative disorders. In our case, the mild clinical presentation as esophageal and gastric vari- defect in antithrombin III levels may also have con- ces. tributed. The fact that this patient was in a thrombo- This case represents an unusual model of portal philic state is also suggested by the clinical manifes- hypertension and suggests that the pathophysiological

Digestive Diseases and Sciences, Vol. 45, No. 2 (February 2000) 375 BERTOLOTTI ET AL

Fig 3. CT portography showing large jejunal varices (arrows). processes and clinical manifestations of increased gastrointestinal bleeding. Am J Gastroenterol 87:514–517, blood pressure in the portal system may be very 1992 similar even if the etiologies and the anatomical sites 4. Kotﬁla R, Trudeau W: Extraesophageal varices. Dig Dis 16:232–241, 1998 of manifestation are quite distinct. Our report en- 5. Valla D, Casadevall N, Huisse MG, Tulliez M, Grange JD, courages wider utilization of jejunoenteroscopy, a Muller O, Binda T, Varet B, Rueff B, Benhamou JP: Etiology relatively noninvasive procedure (16, 17), as a diag- of portal vein thrombosis in adults. A prospective evaluation of nostic tool and the use of angiography even in the primary myeloproliferative disorders. Gastroenterology absence of concurrent bleeding, especially when the 94:1063–1069, 1988 6. Attias E, Smadja C, Vons C, Traynor O, Franco D: Bleeding clinical history of the patient (ie, previous splenec- from intestinal varices after a Warren shunt. J Clin Gastroen- tomy or abdominal surgery in general; presence of a terol 9:585–587, 1987 hematological disorder) may raise the suspicion of 7. Paquet K-J, Lazar A, Bickhart J: Massive and recurrent gas- intestinal varices. We believe that such uncommon trointestinal hemorrhage due to jejunal varices in an afferent events may be increasingly diagnosed as the cause of loop—diagnosis and management. Hepato-Gastroenterol 41:276–277, 1994 gastrointestinal bleeding of, up to now, “unknown” 8. Boughton BJ: Hepatic and portal vein thrombosis. Closely origin. associated with chronic myeloproliferative disorders. BMJ 302:192–193, 1991 REFERENCES 9. Muller EW, De Wolf JTM, Haagsma EB: Portal hypertension as presenting feature of a myeloproliferative disorder. Diagno- 1. Cappell MS, Price JB: Characterization of the syndrome of sis and therapeutic dilemmas. Scand J Gastroenterol 28(suppl small and large intestinal variceal bleeding. Dig Dis Sci 32:422– 200):74–79, 1993 427, 1987 10. Hayag-Barin JE, Smith RE, Tucker FC Jr: Hereditary sphero- 2. Philippakis M, Karkanias G, Sakorafas GH, Pontikis I, Gavalas cytosis, thrombocytosis, and chronic pulmonary emboli: A case M: Massive gastrointestinal bleeding secondary to intestinal report and review of the literature. Am J Hematol 57:82–84, varices. Eur J Surg 158:379–381, 1992 1998 3. Yuki N, Kubo M, Noro Y, Kasahara A, Hayashi N, Fusamoto 11. McGrew W, Avant GR: Hereditary spherocytosis and portal H, Ito T, Kamada T: Jejunal varices as a cause of massive vein thrombosis. J Clin Gastroenterol 6:381–382, 1984

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